A must-read for heart disease patients: mavacamten instructions

1. Common name: mavacamten , mavacamten

Product name: Maifantuo,CAMZYOS

All names: mavacamten

2. Indications:

Mavacamten is indicated for the treatment of adult patients with New York Heart Association (NYHA) Class II-III obstructive hypertrophic cardiomyopathy (HCM) to improve function and symptoms.

3. Usage and dosage:

1.Initiation, maintenance and discontinuation of treatment: Confirm that females of reproductive potential are not pregnant and are using effective contraceptive methods. It is not recommended to start or increase the dose of CAMZYOS in patients with left ventricular ejection fraction (LVEF)<55%.

2. Recommended dosage: The recommended starting dose is 5 mg taken orally once a day, regardless of food; the allowed subsequent doses are 2.5 mg, 5 mg, 10 mg or 15 mg taken orally once a day. The maximum recommended dose is 15 mg orally once daily. Swallow capsule whole. Do not break, open or chew capsules. Daily dosing takes several weeks to achieve steady-state drug levels and therapeutic effects, and genetic variation in metabolism and drug interactions can lead to large differences in exposure.

3. Dosage adjustment: Patients may develop heart failure when taking Mavaceta . Periodic evaluation of LVEF and Valsalva left ventricular outflow tract (LVOT) gradient is required to enable careful titration to achieve an appropriate target Valsalva LVOT gradient while maintaining LVEF ≥50% and avoiding symptoms of heart failure. When initiating or titrating Valsalva, consider first LVEF and then Valsalva LVOT gradient and patient clinical status to guide the appropriate dose of Valsalva. Initiate and maintain appropriate mavacartide dosage and monitoring schedule according to algorithm.

If LVEF is less than 50% while taking Mavakatai, interrupt treatment. Follow the Interrupt Algorithm for guidance on interrupting, restarting or stopping Mavakite. If interrupted at 2.5mg, it will either restart at 2.5mg or be permanently interrupted.

4. Drug interaction: Weak for useFor patients on stable treatment with a CYP2C19 inhibitor or a moderate CYP3A4 inhibitor, take the recommended starting dose of 5 mg orally once daily. For patients starting a weak CYP2C19 inhibitor or a moderate CYP3A4 inhibitor, reduce the dose of mavacartide by one level (i.e., 15 mg → 10 mg; 10 mg → 5 mg; or 5 mg → 2.5 mg). In patients stabilized on therapy with 2.5 mg of mavacate, avoid concurrent use of weak CYP2C19 and moderate CYP3A4 inhibitors because lower once-daily mavacate doses are not available.

4. Adverse reactions:

In clinical studies of Marvaceta, the most common adverse reactions were dizziness, difficulty breathing, systolic dysfunction (the heart's inability to pump with enough force), and syncope (fainting).

5. Supply and storage:

Mavacamten is available in immediate-release Size 2 hard gelatin capsules containing 2.5 mg, 5 mg, 10 mg or 15 mg of mavacamten and stored at 20°C to 25°C (68°F to 77°F), with a tolerance of 15°C to 30°C (59°F to 86°F).

6. Taboo:

Mavaceta is contraindicated in combination with the following medications:

1. Moderate to severeCYP2C19 inhibitor or severe CYP3A4 inhibitor;

2. Moderately strongCYP2C19 inducer or moderately strong CYP3A4 inducer

7. Mechanism of action:

Mavaceta is a selective allosteric and reversible inhibitor of cardiac myosin. Mavacate modulates the number of myosin heads that can enter the "on-actin" (force-producing) state, thereby reducing the probability of force-producing (systolic) and residual (diastolic) cross-bridge formation. Excessive myosin-actin cross-bridge formation and dysregulation of the hyperrelaxed state are mechanistic hallmarks of HCM. Mavacatelshifts the entire myosin population toward an energy-saving, replenishable, super-relaxing state. InHCM patients, myosin inhibition with Mavacatel reduced dynamic LVOT obstruction and improved cardiac filling pressures.

8. Special groups:

1. Women: According to animal data, taking Mavaceta by pregnant women may cause harm to the fetus. Advise females of reproductive potential to use effective contraception during treatment and for 4 months after the last dose. CHCs containing a combination of ethinyl estradiol and norethindrone may be used with Mavacartage. However, mavacate may reduce the effectiveness of certain other combined hormonal contraceptives (CHC). If theseCHCs are used, advise patients to add a non-hormonal contraceptive method (e.g., condoms) during concomitant use and for 4 months after the last dose.

Reference materials:https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=669c936b-3ee6-4e36-8a22-79dd11b1255b